A 20-year-old man with Marfan syndrome was recommended emergency operation for impending rupture of TAA. Because he was in the state of acute heart failure due to coexisting AAE and AR, so the simultaneous operations for AAE, AR and TAA were needed. He underwent Bentall, arch replacement and elephant trunk operation utilizing separate extracorporeal circulation and circulatory arrest. Chest CT, taken after 5 months postoperatively, revealed only a small amount of thrombus in TAA. Anticoagulation therapy stood in the way of aneurysmal thrombo-exclusion. The second operation, grafting of descending thoracic aorta, was performed 6 months later and elephant trunk made the second operation easier. Now 12 months passed after the second operation, he is in good health.